title: Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group.
abstract: BACKGROUND
  Survival data of malignant rhabdoid tumour of the kidney (MRTK) registered in SIOP trials, advocating preoperative chemotherapy, are not available.
  
  
  OBJECTIVE
  To evaluate characteristics, response and survival of MRTK patients registered in recent SIOP protocols.
  
  
  METHODS
  An evaluation of all MRTK patients treated from 1993 to 2005 (SIOP trials 93-01 and 2001) was performed. Data were obtained from study specific case record forms and entered centrally in a database.
  
  
  RESULTS
  Hundred and seven patients were identified (57 male), with a median age at diagnosis of 13 months (interquartile range 6-27 months), and a median follow-up time of 60 months. Left and right kidneys were equally affected. Tumour stage distribution was stage I (6%), stage II (22%), stage III (43%), stage IV (22%) and stage V (3%). Stage IV patients included 17 with pulmonary metastasis (8 lung-only) and 12 with multiple organ metastases (bone, brain and liver). Primary surgery was the upfront treatment approach in 22/107 patients (21%), by which 19 patients reached a complete remission (CR). Median difference in tumour volume before and after preoperative chemotherapy was 69 ml (interquartile range: 4.5-158.0, P < 0.0001), indicating marked chemosensitivity. The 5-year event-free survival (EFS) of the total group was 22% (95% CI: 15-33) and overall survival 26% (95% CI: 18-37). Most events (86%) occurred within the first 2 years after diagnosis. Younger age at diagnosis was an important adverse prognostic factors for survival. In contrast, tumour volume at diagnosis, nor volume reduction was associated with outcome.
  
  
  CONCLUSIONS
  MRTK has a poor outcome especially in young and advanced-stage disease patients. Neither tumour volume at diagnosis, nor pre-operative chemosensitivity are prognostic factors for survival.
authors.affiliation: Department of Pediatric Oncology/Hematology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands. m.vandenheuvel@erasmusmc.nl
authors.forename: Marry M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
authors.forename: Harm
authors.initials: H
authors.lastname: van Tinteren
authors.forename: Harriet
authors.initials: H
authors.lastname: Rehorst
authors.forename: Aurore
authors.initials: A
authors.lastname: Coulombe
authors.forename: Catharine
authors.initials: C
authors.lastname: Patte
authors.forename: Beatriz
authors.initials: B
authors.lastname: de Camargo
authors.forename: Jan
authors.initials: J
authors.lastname: de Kraker
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.forename: Rieneke
authors.initials: R
authors.lastname: Lugtenberg
authors.forename: Kathy
authors.initials: K
authors.lastname: Pritchard-Jones
authors.forename: Bengt
authors.initials: B
authors.lastname: Sandstedt
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Norbert
authors.initials: N
authors.lastname: Graf
authors.forename: Gordan M
authors.initials: GM
authors.lastname: Vujanic
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003609:Dactinomycin / Q000627:therapeutic use; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000401:mortality* / Q000473:pathology; D008297:Male; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D012042:Registries; D018335:Rhabdoid Tumor / Q000188:drug therapy* / Q000401:mortality* / Q000473:pathology; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D003609:Dactinomycin; D014750:Vincristine